Citation, DOI and article data
Scleromalacia perforans (or non-inflammatory necrotizing scleritis) is a severe and very rare form of scleritis.
- rheumatoid arthritis
- Crohn disease
- ulcerative colitis
- systemic lupus erythematosus
- Behçet disease 5
- relapsing polychondritis 5
- granulomatosis with polyangiitis 3
- rhinosporidiosis 4
The condition is generally asymptomatic. It is usually found on visual inspection during a formal eye examination.
Scleromalacia perforans is a rare non-inflammatory form of anterior necrotizing scleritis, usually seen in patients with autoimmune disease.
The scleral thinning may secondarily calcify, and can be seen on CT. In addition the rare sequela of atraumatic globe rupture might be . Other manifestations of the underlying autoimmune disease may also be present.
Treatment and prognosis
The condition is usually asymptomatic. Rupture of the globe is rare and usually only seen in those with coexisting markedly elevated intraocular pressure, i.e. severe glaucoma 1.
Historically the condition has shown a limited response to conventional treatments, e.g. steroids. A recent article suggests that adalimumab may be of benefit 2.
- 1. Wu CC, Yu HC, Yen JH, Tsai WC, Liu HW. Rare extra-articular manifestation of rheumatoid arthritis: scleromalacia perforans. (2005) The Kaohsiung journal of medical sciences. 21 (5): 233-5. doi:10.1016/S1607-551X(09)70193-7 - Pubmed
- 2. Lawuyi LE, Gurbaxani A. Refractory necrotizing scleritis successfully treated with adalimumab. (2016) Journal of ophthalmic inflammation and infection. 6 (1): 37. doi:10.1186/s12348-016-0107-y - Pubmed
- 3. Reddy SC, Tajunisah I, Rohana T. Bilateral scleromalacia perforans and peripheral corneal thinning in Wegener's granulomatosis. (2011) International journal of ophthalmology. 4 (4): 439-42. doi:10.3980/j.issn.2222-3959.2011.04.22 - Pubmed
- 4. Bothra N, Mittal R, Tripathy D. Scleromalacia Perforans from Long-Standing Bulbar Conjunctival Rhinosporidiosis. (2016) Ophthalmology. 123 (7): 1493. doi:10.1016/j.ophtha.2016.04.010 - Pubmed
- 5. Sakellariou G, Berberidis C, Vounotrypidis P. A case of Behcet's disease with scleromalacia perforans. (2005) Rheumatology (Oxford, England). 44 (2): 258-60. doi:10.1093/rheumatology/keh445 - Pubmed